Frequency of Diabetes Mellitus in Patients with Klinefelter's Syndrome of Different Chromosome Constitutions and the XYY Syndrome. Plasma Insulin and Growth Hormone Level After a Glucose Load

Nielsen, Jørgen G.; Johansen, Klaus; Yde, Hans

doi:10.1210/jcem-29-8-1062

Cited by 70 publications

(41 citation statements)

References 19 publications

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“…2) After admission, we studied his insulin sensitivity using the glucose clamp test (high insulinemic normal glycemic clamp study Amongthose cases, 61 patients (6.8%) were described as having diabetes mellitus ( is higher than that in the normal population, which may in part arise from the complication of diabetes (32). The frequency of diabetes mellitus in patients with Klinefelter's syndrome has been reported as 15%-50% in Western countries (3,33,34), while, in Japan it was lower at 3.9%-4.1% (6, 7). Sagara et al suggested that the low prevalence of diabetes in Japanese with Klinefelter's syndrome might be caused by insufficient examinations for glucose tolerance (6).…”

Section: Case Reportmentioning

confidence: 99%

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Diabetes Mellitus Associated with Klinefelter's Syndrome: A Case Report and Review in Japan.

et al. 2002

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Wereport a case of Klinefelter's syndrome in a 48-yearold man who had diabetes mellitus associated with severe insulin resistance. Wediagnosed him with Klinefelter's syndrome from his atrophic testicles, primary hypogonadism in hormonal examination, and a chromosomalaberration of 47,XXY. He showed severe decreased insulin sensitivity in a hyper-insulinemic euglycemic clamp test. He had injected over 100 units of insulin per day, however, testosterone replacement and administration of pioglitazone improved his glycemic control, which resulted in a decrease of insulin dose to less than 50 units per day. Here, we discuss the characteristics of diabetes mellitus associated with Klinefelter's syndrome in Japanese patients including this case. (Internal Medicine 41: 842-847, 2002)

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Section: Case Reportmentioning

confidence: 99%

“…It was reported that patients with Klinefelter's syndrome tended to have diabetes mellitus or autoimmune diseases (3)(4)(5). It has not been clarified why diabetes mellitus develops in patients with Klinefelter's syndrome, and what the characteristics of diabetes in the syndrome are.…”

Section: Introductionmentioning

confidence: 99%

Diabetes Mellitus Associated with Klinefelter's Syndrome: A Case Report and Review in Japan.

et al. 2002

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“…[8] In Western countries, incidence of diabetes in patients with KS has been reported as 15% to 50%. [9][10][11] In this syndrome, type and severity of diabetes may differ, and many factors may contribute to development of diabetes. Insulin resistance is an important component of diabetes in this syndrome.…”

Section: Discussionmentioning

confidence: 99%

The Effects of Androgen Replacement Therapy on Glycemic Control in a Case with Klinefelter Syndrome and Poorly Controlled Diabetes

Özderya¹

2017

South Clin Ist Euras

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Klinefelter syndrome (KS) is the most common sex chromosome anomaly among men. It is usually characterized by hypergonadotropic hypogonadism, eunuchoid body structure, and most often, 47,XXY karyotype. Studies have shown that KS patients have high risk of developing autoimmune diseases and diabetes, and the hypogonadism that accompanies the syndrome makes regulation of diabetes more difficult. A 39-year-old man with KS was admitted to Endocrinology and Metabolic Diseases Polyclinic because of increased blood glucose level: His glycated hemoglobin (HbA1c) value was 14.9%. He had diabetes for approximately 5 years and was using 80 IU insulin daily at time of admission, but after testosterone replacement therapy, his insulin requirement decreased to 60 units. Follow-up HbA1c was 7.7% after 2 months of androgen replacement therapy. Hypoandrogenemia may contribute to development of newly diagnosed diabetes or deterioration of pre-existing diabetes. Testosterone replacement therapy can improve glycemic control in these patients.

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“…A high incidence of diabetes mellitus was reported in Klinef elter's syndrome (Nielsen et al, 1969) but the association of polycythemia vera with this syndrome is rare in the literature. Geraedts et al (1980) reported a patient with a 47,XXY karyotype who developed polycythemia very at age below 40 and later acute myelomonocytic leukemia.…”

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confidence: 98%

A chromosome study in a case of Klinefelter's syndrome associated with diabetes mellitus and polycythemia vera.

Hoshino

Nakada

Ishitobi

et al. 1982

Proceedings of the Japan Academy. Ser. B: Physical and Biologic

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(Communicated by Saj iro MAKINO, M. J. A., May 12, 1982) It has been known that Klinefelter's syndrome is associated occasionally with myeloprolif erative disorders, particularly in a majority of cases with different forms of leukemia. The literature refers to only one case of polycythemia vera in association with this syndrome (Geraedts et al., 1980) . In this paper are reported the chromosomes of a male patient who had symptoms and signs of Klinef elter's syndrome with diabetes mellitus and developed later polycythemia vera.Case report. The patient was born in 1913 between a 48-yearold father and a 29-year-old mother. He married at the age of 26 years, but has no children. He is the third of five siblings, and all the siblings have children except the youngest brother who died in the World War II. There is no consanguinity in his family. Diabetes mellitus was diagnosed at the age of 50 years. At the age of 62 years, conj unctival congestion and erythema of the hands were noted. His red blood cell count was found to be markedly elevated at the age of 65 years. Then he was referred to our clinic due to polycythemia and diabetes mellitus.On physical examinations the patient was 171 cm tall, weighed 65 kg, and had an arm span of 175.5 cm (Fig. 1). His skin was deep red in color on the cheeks, lips, ears, tip of the nose and distal portions of the extremities. Bulbar and palpebral conjunctivae were markedly congested. He had bilateral gynecomastia and cubitus valgus, and a simian crease on the right hand. The axillary and pubic hair was scanty, and the testes were small measuring 2.5 by 1.5 cm on both sides. Hepatosplenomegaly was noted.Hematological examinations revealed RBC 938 x 104/cmm, Hb 24.0 g/dl, Ht 79.2% and WBC 12,400/cmm with 78% neutrophils and 8 % eosinophils. Platelet count was 7.2 x 104/cmm, while it was 45.3 x 104/cmm after a series of phlebotomy totaling 740 ml. Erythrocyte sedimentation rate was 0 mm in one hour. Blood chemistry

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Frequency of Diabetes Mellitus in Patients with Klinefelter's Syndrome of Different Chromosome Constitutions and the XYY Syndrome. Plasma Insulin and Growth Hormone Level After a Glucose Load

Cited by 70 publications

References 19 publications

Diabetes Mellitus Associated with Klinefelter's Syndrome: A Case Report and Review in Japan.

Diabetes Mellitus Associated with Klinefelter's Syndrome: A Case Report and Review in Japan.

The Effects of Androgen Replacement Therapy on Glycemic Control in a Case with Klinefelter Syndrome and Poorly Controlled Diabetes

A chromosome study in a case of Klinefelter's syndrome associated with diabetes mellitus and polycythemia vera.

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